Interstitial Lung Diseases in Collagen Vascular Diseases

Sung Hwan Jeong

doi:10.5124/jkma.2009.52.1.30

J Korean Med Assoc > Volume 52(1); 2009 > Article

Jeong: Interstitial Lung Diseases in Collagen Vascular Diseases

Focused Issue of This Month

Interstitial Lung Diseases

Journal of the Korean Medical Association

Journal of the Korean Medical Association 2009; 52(1): 30-40.

Published online: January 31, 2009

DOI: http://dx.doi.org/10.5124/jkma.2009.52.1.30

Interstitial Lung Diseases in Collagen Vascular Diseases

Sung Hwan Jeong, MD

Department of Internal medicine, Gachon University of Medicine and Science, Korea. jsw@gilhospital.com

Abstract

Different types of interstitial lung diseases (ILDs) develop in collagen vascular diseases (CVDs) such as scleroderma, rheumatoid arthritis, systemic lupus erythematosus, dermatopolymyositis, Sjogren's syndrome, and mixed connective tissue disease. These CVDs represent various histological patterns, including usual interstitial pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, bronchiolitis obliterance organizing pneumonia, diffuse alveolar damage, and bronchiolitis. The clinical presentations, prognosis, and response to treatment vary depending on the underlying CVDs, as well as histological patterns of ILDs. In general, the prognosis and survival rate of ILDs in CVDs are better than idiopathic ILDs. Optimal treatment also varies depending on the type of CVDs and the presence of interstitial lung disease, although in many cases, a combination of corticosteroids and cytotoxic drugs are given.

Collagen vascular disease; Interstitial lung disease; Fibrosis

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Figure 1

Pulmonary hypertension in systemic sclerosis in a 46-year-old woman.

Figure 2

Fibrosing alveolitis in systemic sclerosis, in a 46-year-old woman.

Figure 3

RA with fibrosing alveolitis in a 47-year-old woman. CT scan shows honeycombing, irregular linear hyperattenuating areas in the basal areas of both lungs.

Figure 4

RA with NSIP in a 48-year-woman. CT scan shows patchy distribution of irregular linear hyperattenuating areas and ground glass attenuation in both lungs.

Figure 5

RA with necrobiotic nodule in a 47-year-old woman. High-resolution computed tomography shows a rheumatoid nodules located in the both lungs.

Figure 6

Systemic lupus erythematosus and acute lupus pneumonitis CT scans (3-mm collimation) obtained with lung window at the levels of the aortic arch (A) and lingular segmental bronchus (B) show airspace consolidation in the right upper lobe and ground-glass attenuation in the left upper lobe. Bronchial dilatation is seen in the consolidation in the right upper lobe (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine.

Figure 7

SLE with NSIP in a 42-year-old woman. CT scan shows patchy distribution of irregular linear hyperattenuating areas and ground glass attenuation with traction bronchiectasis in both lungs.

Figure 8

Dermatopolymyositis. High-resolution computed tomography at the level of the hila shows a bronchiolitis obliterans with organizing pneumonia pattern (ERJ 2001; 18: S32, 69s-80s).

Figure 9

Dermatomyositis and usual interstitial pneumonia (A, B) CT scans obtained at the levels of the bronchus intermedius (A) and liver dome (B) show patchy areas of ground-glass attenuation and irregular linear hyperattenuating areas with random distribution in both lungs. (C) Photomicrograph (original magnification, × 12.5; H-E stain) shows irregular interstitial fibrosis (arrows) and mononuclear cell infiltration with subpleural predominance. Germinal center formation is also seen (arrowheads) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine.

Figure 10

Sjögren's syndrome and lymphoid interstitial pneumonia (A) CT scan shows centrilobular nodules and branching linear structures (straight arrow) in the right lung. Many thin-walled cysts (curved arrows) were seen in both lungs. (B) Photomicrograph (original magnification, × 20; H-E stain) shows diffuse lymphocyte infiltration in the peribronchovascular interstitium and surrounding alveolar septa (arrows) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine.

Figure 11

Mixed connective tissue disease and usual interstitial pneumonia. CT scan shows subpleural areas of ground-glass attenuation, irregular linear hyperattenuating areas, and traction bronchiectasis and bronchiolectasis (arrows) (RadioGraphics 2002; 22: S151-S165). Images by courtesy of Kyung Soo Lee, MD; Samsung Medical Center, Sungkyunkwan University School of Medicine.